Phase 2

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Neurology Part 2

• Phase 2
• Kaveesha Rajapaksa
• Ryad Chebbout

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Aims

• Pathology, Aetiology, Clinical Symptoms and
  Signs, Investigations and Management of
• Epilepsy
• Multiple Sclerosis
• Guillain-Barre Syndrome
• Motor Neuron Disease
• Parkinson's Disease
• Dementia

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Epilepsy

• Definition
• Recurrent tendency to have spontaneous,
  intermittent and abnormal electrical activity in
  a part of the brain or generalised across the
  brain. Leading to seizures.

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Epilepsy

• Aetiology
• Idopathic (2/3rds)
• Structural (Trauma, SOL, Developmental, Stroke)
• Metabolic (hypoglycaemia, hypocalcemia,
  hyponatraemia)

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Epilepsy

• Partial focal onset (localising features!)
• Generalised no localising features!
• Simple aware
• Complex impaired awareness

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Epilepsy

• Partial Seizures
• Commonly caused by structural pathology.
• Simple Partial (Jacksonian March)
• Complex Partial classically temporal lobe!
• Partial Secondary Generalisation

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Epilepsy

• Generalised Seizures
• Commonly idiopathic.
• Absence lt/ 10sec, childhood
• Tonic-Clonic complex, stiff-gtjerk, post-ictal
  confusion drowsiness
• Myoclonic sudden limb/face/trunk jerk
• Atonic loss of tone-gtfall, no LOC

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Epilepsy

• Prodrome Aura Ictal Post-Ictal
• Prodrome
• Mood/Behaviour Change

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Epilepsy

• Aura
• Strange Smell, Flashing Lights, Déjà vu/Jamais vu
• Post-Ictal
• Headache, Confusion, Myalgia, Sore Tongue, Todds
  Palsy (hemiplegia), Dysphasia

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Epilepsy

• Ictal Localising Features
• Temporal Lobe Automatisms (lip
  smacking/fiddling), Visceral Aura (abdominal
  rising sensation), Dysphasia, Déjà vu/Jamais Vu,
  Hallucinations
• Frontal Lobe Jacksonian March (tingling/muscle
  contractions from fingers to ipsilateral face),
  Todds Palsy

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Epilepsy

• Parietal Lobe Tingling Numbness
• Occipital Visual Phenomena (spots/lines/flashes)

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Epilepsy

• Investigations
• EEG (classification)
• MRI (structural lesions)
• MEG/PET/SPECT ictal (localise epileptogenic focus
  for surgery)

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Epilepsy

• Management
• Partial Carbamazepine
• Generalised Sodium Valproate OR Lamotrigine
• Neurosurgical Resection

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Epilepsy

• Management
• Counselling employment, insurance, driving (1yr
  seizure free)
• Contraception and pregnancy
• Epilepsy Nurse Specialist

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Epilepsy

• Complications
• Sudden Unexpected Death in Epilepsy (SUDEP)
• Status Epilepticus

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Multiple Sclerosis

• Discrete Plaques of Demyelination in Central
  Nervous System
• T-cell Mediated

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Multiple Sclerosis

• Risk Factors Women, Temperate Areas
• 30yrs

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Multiple Sclerosis

• Demyelination Plaque (commonly periventricular,
  cervical spine, brain stem)
• Heals Incompletely
• Prolonged Demyelination
• Axonal Loss
• Clinically Progressive Symptoms

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Multiple Sclerosis

• Clinical Courses
• Benign
• Relapsing Remitting
• Secondary Progressive
• Primary Progressive

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Multiple Sclerosis
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Multiple Sclerosis

• Symptoms
• Monosymptomatic!
• Disseminated in Time and Space

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Multiple Sclerosis

• Optic Neuritis Decreased Visual Acuity, Pain on
  Eye Movement, Dyschromatopsia
• Sensory Lhermittes Sign, Limb Numbness/Tingling
• Motor Transverse Myelitis, UMN Weakness,
  Uhthoffs Phenomenon

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Multiple Sclerosis

• Other Ataxia, Erectile Dysfunction, Urinary
  retention

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Multiple Sclerosis

• Investigation
• Dissemination in Time and Space!
• Clinically (attacks clinical lesions, 22, 21,
  11)
• /- Aid of MRI
• CSF (Oligoclonal Bands)
• Evoked Potentials

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Multiple Sclerosis

• Management
• Acute Methylprednisolone IV
• Chronic Interferon/Glatiramer, Natalizumab

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Guillain-Barre Syndrome

• Acute AUI Inflammatory Demyelinating
  Polyneuropathy
• Triggers Campylobacter jejuni, CMV, Mycoplasma

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Guillain-Barre Syndrome

• Symptoms
• 4 wk peak!
• Weakness Leg, Trunk, Respiratory. Proximal,
  Distal. Symmetrical.
• Back/Limb Pain
• Autonomic Features seating, tachycardia,
  arrhythmias.

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Guillain-Barre Syndrome

• Signs
• Areflexia!

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Guillain-Barre Syndrome

• Investigations
• Nerve Conduction Studies slow conduction
• Regular FVC

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Guillain-Barre Syndrome

• Management
• IV Immunoglobin or Plasma Exchange

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Motor Neuron Disease

• Selective loss of motor neurons in Motor Cortex
  (UMN), Cranial Nerve Nuclei (UMN/LMN) and
  Anterior Horn Cells (LMN).
• gt40yrs.
• lt/10 Familial. 20 SOD1 mutation.

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Motor Neuron Disease

• Key Features
• UMN LMN Signs
• No Sensory Loss/Sphincter Disturbance
• No Eye Involvement

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Motor Neuron Disease

• Clinical Patterns
• Amyotrophic Lateral Sclerosis
• Progressive Bulbar Signs CN IX-XII,
  bulbar/pseudobulbar palsy
• Progressive Muscular Atrophy no UMN signs
• Primary Lateral Sclerosis mainly UMN

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Motor Neuron Disease

• Associations
• Fronto-Temporal Dementia (10-35)!

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Motor Neuron Disease

• Signs
• Stumbling Spastic Gait
• Foot Drop /- Proximal Myopathy
• Weak Grip
• Spasticity/Hypereflexia/Upwards Plantars
  Wasting/Fasciculations

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Motor Neuron Disease

• Investigations
• MRI/LP/Neurophysiology exclude other causes
• Management
• Riluzole prolong life by 3months
• Symptomatic MDT (Ventilation, PEG)

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Parkinson's Disease

• Decreased Dopaminergic Neurons in Substantia
  Nigra (Pars Compacta)
• Decreased Striatum Dopamine Levels
• Decreased Basal Ganglia Cortex Communication
• Decreased Movement

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Parkinson's Disease

• 65yrs. Associated with Lewy Bodies

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Parkinson's Disease

• Classic Triad
• Tremor resting, pill-rolling
• Rigidity increased tone, cogwheel rigidity,
  leadpipe rigidity
• Bradykinesia slowness of movement initiation
• Expressionless Face, Monotonous Hypophonic
  Speech, Micrographia
• Gait Festinant, Reduced Arm Swing

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Parkinson's Disease

• Other Symptoms
• Anosmia
• Depression
• Sleep disturbances
• Visual Hallucinations (animals,
  children)Dementia (late stage)

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Parkinson's Disease

• Management
• L-Dopa Dopa-Decarboxylase Inhibitor (e.g.
  Madopar)
• Efficacy reduces with time Increased Dose
• Dyskinesias, Off Freezing, End-of-Dose Reduced
  Relapse

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Parkinson's Disease

• Management
• Dopamine Agonist Ropinirole/Pramipexole
• Apomorphine potent DA agonist, acute
• Anticholinergics tremor
• Deep brain stimulation

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Dementia

• Syndrome of progressive deficits in 2 or more
  higher cognitive domains. (Memory, language,
  apraxia, agnosia, visuospatial function,
  personality)
• Interferes with social functioning.
• Occurs in clear consciousness.
• gt80yrs20, gt100yrs70

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Dementia

• Types
• Alzheimer's Disease
• Vascular Dementia
• Lewry Body Dementia
• Fronto-Temperal Dementia

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Alzheimer's Dementia

• Increased Beta-Amyloid Peptide Progressive
  Neuronal Damage (hippocampus, amygdala, temporal
  neocortex)
• Neurofibillary Tangles
• Amyloid Plaques
• Decreased Ach

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Alzheimer's Dementia

• Risk Factors
• Family History, Downs Syndrome, Homzygosity for
  ApoE e4 Allele, DM/HTN/AF
• Protective Factors
• Smoking, Oestrogen

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Alzheimer's Dementia

• Symptoms
• Progressive Global Cognitive Impairment
• Aphasia
• Anosognosia (lack of insight)
• Irritability
• Mood Disturbance Depression, Euphoria
• Behavioural Change Wandering, Aggression

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Alzheimer's Dementia

• Investigations
• MMSE or Addenbrooks Cognitive Exam
• CT temporal/parietal atrophy, ventricular
  enlargement
• MRI hippocampus/amygdala/medial temporal lobe
  grey matter atrophyCSF phosphorylated tau
  protein

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Alzheimer's Dementia

• Management
• Acetylcholinesterase Inhibitors (Donepezil,
  Rivastigmine, Galantamine) help lay down new
  memories
• Memantine (Antiglutamatergic)
• BP Control

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Vascular Dementia

• Cumulative effect of many small strokes.
• Vascular RFs Stroke Hx, HTN
• Sudden Onset Stepwise Deterioration
• Emotional/Personality Changes
• Cognitive Defecits
• Depression/Labile Mood

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Lewy Body Dementia

• Fluctuating Cognitive Impairment
• Visual Hallucinations (animals/children) /-
  Parkinsonism
• Repeated Falls/Syncope
• CT/MRI relative sparing of medial temporal lobe
• Histology Lewy Bodies in Brainstem/Neocortex

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Fronto-Temporal Dementia

• Frontal Temporal Atrophy. AD Histology.
• Early, 45-65yrs
• Behavioural/Personality Change
• Disinhibition
• Change in Diet (sweets, overeating)
• Emotional Blunting
• Pick bodies on histology

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Dementia

• Management
• Care Coordinator
• Capacity
• Develop Routines, Plan Ahead
• Challenging Behaviour Lorazepam, Risperidone
  (not Lewy Body!)
• Depression - Citalopram

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• A 65 year old man with walking difficulties
  presented to his general practitioner. He
  complained of worsening tremor in the right arm
  with stiffness, which he said on occasion spread
  to his right leg. He said that the tremor was
  much worse when he was stressed, or in public.
  Examination revealed a man with a resting tremor,
  marked cogwheeling rigidity of the right side and
  an inability to perform repetitive tasks with the
  right arm. His gait was not normal and he had a
  reduced arm swing on the right.

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• A 36 year old male patient presents with
  increasing unsteadiness which started two days
  ago. Two years ago he had blurred vision in his
  left eye which improved considerably within a few
  weeks, but left him with some minor deficit.
  Eight years ago he had a 3 week episode of
  numbness in his left arm.